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Most cases are sporadic; however, the most frequent chromosomal abnormality (occurs in 60%) is deletion or rearrangement of 3p21-26. The von Hippel-Lindau gene has been mapped to that region and appears to have ubiquitin ligase activities that influence regulation of speed of transcription and turnover of damaged proteins. It is unclear how lesions in the gene lead to cancer.

Outline

Section 6. Hematology and Oncology